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中间型地中海贫血的高凝状态。

The hypercoagulable state in thalassemia intermedia.

作者信息

Taher Ali T, Musallam Khaled M, Inati Adlette

机构信息

Department of Internal Medicine, Hematology-Oncology Division, American University of Beirut Medical Center, Beirut, Lebanon.

出版信息

Hemoglobin. 2009;33 Suppl 1:S160-9. doi: 10.3109/03630260903351619.

Abstract

Although the life expectancy of beta-thalassemia patients has markedly improved over the last few years, patients still suffer from many complications of this congenital disease. The presence of a high incidence of thromboembolic events, mainly in beta-thalassemia intermedia, has led to the identification of a hypercoagulable state in these patients. In this review, the molecular and cellular mechanisms leading to hypercoagulability in beta-thalassemia are highlighted, with a special focus on thalassemia intermedia being the group with the highest incidence of thrombotic events as compared to other types of thalassemia. Recommendations for thrombosis prophylaxis in these patients are also discussed.

摘要

尽管在过去几年中,β地中海贫血患者的预期寿命有了显著提高,但患者仍遭受这种先天性疾病的许多并发症困扰。血栓栓塞事件的高发生率,主要见于中间型β地中海贫血患者,这使得人们认识到这些患者存在高凝状态。在本综述中,重点阐述了导致β地中海贫血患者高凝状态的分子和细胞机制,特别关注中间型地中海贫血,因为与其他类型的地中海贫血相比,该型患者发生血栓事件的发生率最高。还讨论了对这些患者进行血栓预防的建议。

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